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Dr. Wael Gabr
NEUROPATHIES
Dr. Wael Gabr
START WITH WHY
ANATOMY
CNS PNS
UMNL= lesion above
LMNL= lesion below
PNS CONSISTS
OF?
1. Anterior horn cell
2. Motor neurons
3. Ventral root
4. Dorsal root ganglia
1. Dorsal root
2. Plexus
3. Peripheral nerve
4. Neuromuscular
junction
5. Muscles
NERVE
ANATOMY
WHAT IS THE MAIN FUNCTION
OF THE NERVE??
WHAT IS THE NERVE
FUNCTION?
Conduction of impulse????????????
TYPES OF NERVE
CONDUCTION
NERVE FIBERS
NERVES DIAMETER
PATHOGENIC
MECHANISMS
OF NERVE
DAMAGE
A. Wallerian degeneration
B. Axonal degeneration
C. Segmental degeneration
WALLERIAN
DEGENERATI
ON
Dying forward
phenomenon
AXONAL
DEGENERATIONDying back phenomenon (Typically in metabolic Neuropathies)
SEGMENTAL
DEGENERATIO
N
(DEMYELINATIO
N)
Ch. by focal degeneration
of the myelin with sparing
of the axons and bounded
on each end by preserved
myelin
CLINICAL PICTURE
Sensory, Motor & Autonomic
SENSORY SYMPTOMS
MOTOR SYMPTOMS
MOTOR SYMPTOMS
AUTONOMIC
NEUROPATHY
Hypertension (+ve)
Postural hypotension,
Sweating,
Urine retention,
Impotence,
Vascular color changing,
GIT complains.
DEFINITIONS
• Mononeuropathy
• Mononeuritis
multiplex
• Radiculopathy
• Plexopathy
• Cranial neuropathy
• Polyneuropathy
(peripheral
neuropathy)
ETIOLOGY OF
POLYNEUROPATHY
Genetics
Medical
IllnessDeficienci
es
Endocrine
Toxic
inflammat
ory
Infectious
Paraprotei
ns
Unknown
CLASSIFICATION
• By type of fiber involved
• Pure sensory
• Pure motor
• Mixed sensory & motor
• Autonomic
• By pathology
• Axonal
• Demyelinating
• Mixed
• By pathology
• Acute (days-1m)
• Subacute (1-2m)
• Chronic (>2m)
• Monophonic, Progressive,
Relapsing
Predominantly
Sensory
• DM & Uraemia
• thiamine deficiency
• leprosy
• HSN & malignancy
• Amyloidosis
• Alcoholic
Predominantly Motor
• GBS & CIDP
• lead
• porphyria
• diphtheria
• botulism
• Charcot-Marie-Tooth
• Paraneoplastic
syndrome
DEMYELINATING
NEUROPATHIES
Acute
• Guillain Barre
syndrome (GBS)
Chronic
• CIDP
• paraproteinemia related
• amiodarone toxicity
• Refsum’s disease
• Charcot-Marie-Tooth
(CMT)
• metachromatic
leucodystrophy
CHRONIC AXONAL
NEUROPATHY
1. Drug and toxin: Alcohol, vincristine, phenytoin,
organophosphate, statins, metronidazole, dapsone
2. Infection: Leprosy, HIV, Borreliosis
3. Connective tissue: Sjogren's syndrome, SLE, rheumatoid arthritis
4. Metabolic: Diabetes, chronic renal failure
5. Paraneoplastic: Carcinoma of the lung and ovary.
6. Inherited: CMT 2 and CMT X
7. Vitamin deficiency: B12, Folic acid, Vitamin E
8. Endocrine: Hypothyroidism
9. Paraproteinemia: Myxedema, Benign monoclonal gammopathy
FOCAL MULTI FOCAL
NEUROPATHIES1. Entrapment neuropathy: Carpal tunnel syndrome,
ulnar nerve at the elbow, common peroneal at the
fibular head
2. Endocrinal: diabetes mellitus, myxedema,
acromegaly
3. Amyloidosis
4. Hereditary neuropathy susceptible to pressure
palsy
5. Vasculitis
6. Multifocal motor neuropathy with conduction block
THICKENED NERVES IN
NEUROPATHY
INVESTIGATIO
NS
1. Nerve Conduction
study
2. Nerve biopsy,
3. Lab. Tests
4. Genetic testing,
5. CSF
GUILLAIN-BARRÉ
SYNDROME
GUILIN-BARRE
SYNDROME
Etiology- C/P- investigations-DD - complication-
Management
CIDP
Immune mediated demyelinating polyneuropathy
Presents with LL weakness and sensory changes, and less
commonly, CN palsy,
One of three clinical syndromes:
1. Monophasic illness with symptoms lasting > 2 months
2. Slow progressive course
3. Relapsing/remitting course
NCS consistent with demyelination
LP with increased protein
Treatment:
CHARCOT-MARIE-TOOTH
DISEASE
 AD, duplication in the peripheral myelin protein 22 (PMP22) gene on 17p11.2-12
 Progressive distal polyneuropathy
 Begins in intrinsic foot muscles, and then progresses to include proximal weakness, as well as to involve hand
muscles
 Hammer toes, pronounced arch, thin tapered appearance of legs
 Onset in first 2 decades
 First symptoms include:
 Difficulty with heel walking
 Pes cavus
 Calf atrophy
 Decreased reflexes
 Progresses to include
 Worsening of above
 Impairment of vibration and proprioception
 Touch, pain and temperature sensation less commonly involved
 Diagnosis: EMG/NCS Genetic testing
CARPAL TUNNEL
SYNDROME
ANY QUESTIONS
MYASTHENIA
MYASTHENIA
ANY
QUESTIONS?
Poly neuropathy in pictures

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Poly neuropathy in pictures

Notes de l'éditeur

  1. Myelin sheath is the most susceptible to damage, It can break down as a result of damage to Schwann cell, myelin itself or disease affecting axon.
  2. Mononeuropathy Pattern of weakness and sensory loss conforms to the distribution of a single nerve Carpal tunnel syndrome Peroneal palsy at the fibular head Mononeuritis multiplex Multiple nerves affected in a random pattern Acute onset, frequently painful Diabetes mellitus, vasculitis Radiculopathy – nerve root Plexopathy – brachial or lumbosacral plexus Cranial neuropathy – Bell’s Palsy, 3rd nerve palsy Polyneuropathy (peripheral neuropathy) Distal, symmetric
  3. Monophasic==> GBS Progressive ==>DM, CIDP Relapsing==> recurrent GBS
  4. Leprosy, Neurofibromatosis, Refsum's disease, Amyloidosis, Roussy Levy syndrome. CMT1,3
  5. Indication of nerve biopsy ( suspicion of) Amyloidosis Vasculitis Leprosy Sarcoidosis Cidp